Therapy of focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is one of the leading courses of idiopathic nephrotic syndrome in adults. It is a histological diagnosis rather than a disease and characterized by damage to podocytes. It occurs as a primary form or as a secondary response to glomerular injury. Distinguishing between primary and secondary forms is important because immunosuppressive therapy is not indicated in patients with secondary FSGS. Patients with secondary FSGS often present with non-nephrotic proteinuria and untreated patients with nephrotic syndrome have a poor renal prognosis. Prognostic factors that influence renal survival of patients with FSGS include the degree of proteinuria, renal dysfunction, histological findings and the response to steroid therapy. There are no randomized controlled trials comparing steroids or other agents to placebo for initial therapy of primary FSGS; however, first line treatment of nephrotic patients with primary FSGS is high dose steroids. Steroid resistance is defined by persistence of the nephrotic syndrome after a more prolonged course of steroid therapy over more than 4 months. Alternative therapies include treatment with calcineurin inhibitors, mycophenolate mofetil, cyclophosphamide and rituximab. © 2013 Springer-Verlag Berlin Heidelberg.